Videolaryngoscopy Bails Us out of Difficult Intubation Scenarios In

Doi: 10.5152/TJAR.2020.00947 Case Report Paediatric Anaesthesia

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Videolaryngoscopy Bails us out of Difficult Intubation Scenarios in Syndromic Children: A Case Series Anju Gupta1 , Nishkarsh Gupta2 , Geeta Kamal3 , Kiran Kumar Girdhar1 1Department of Anaesthesiology, Pain Medicine and Critical Care , AIIMS, New Delhi, India 2Department of Onco-Anaesthesiology and Palliative Medicine, DRBRAIRCH, AIIMS, New Delhi, India 3Department of Anaesthesiology, Chacha Nehru Bal Chikitsalya, New Delhi, India

Cite this article as: Gupta A, Gupta N, Kamal G, Gridhar KK. Videolaryngoscopy Bails us out of Difficult Intubation Scenarios in Syndromic Children: A Case Series. Turk J Anaesthesiol Reanim DOI: 10.5152/TJAR.2020.00947.

Abstract

Airway management in children with craniofacial anomalies can be complicated and may require multiple attempts with conventional direct laryngoscopy (DL). Videolaryngoscopes (VLs) have a well-established role in difficult airway management in adults; however, their role remains to be fully elucidated in paediatric age group. There is a relative paucity in the literature regarding the role of VLs in cases of syndromic children, and it is not clear whether they should be used as an initial option or as a rescue device. Herein, we report a series of cases of children with Pierre Robin sequence, Beckwith–Wiedemann syndrome, and Hurler’s syndrome wherein VLs proved beneficial after multiple failed DL attempts. Following initial failed attempts to intubate using DL, these children were subsequently intubated using VLs. Therefore, VLs should be used for initial intubation attempts in syndromic children with potential difficult airways.

Keywords: Airway management, child, congenital abnormality, intubation, videolaryngoscopy

Introduction

Airway management is a crucial skill for an anaesthesiologist and a fundamental part of general anaesthesia (GA). Paediatric tracheal intubations may be difficult and require multiple attempts, especially in those with craniofacial anomalies (1, 2); however, each additional attempt may increase the airway morbidity (3). There is a growing interest for videolaryngoscopes (VLs) among paediatric anaesthesiologists since it has the potential to facilitate endotracheal intubation and decrease adverse consequences (4, 5). Although the use of VLs has become more prevalent in paediatric anaesthesia practice, documentation of its use, especially for rescue in syndromic children with craniofacial anomalies, remains scarce. This case series describes children with craniofacial anomalies posted for emergency surgery where the use of VLs helped us successfully manage ‘difficult-to-intubate’ scenarios.

Case Presentations

Written informed consent was obtained from the parents of all the three following cases.

Case 1 A 2-day-old, 1.8-kg full-term Pierre Robin sequence (PRS) neonate with micrognathia, retrognathia, glossoptosis, high-arched palate and right-hand deformity presented for tracheoesophageal fistula (TEF) repair (Figure 1). The child was anaesthetised using incremental induction of sevoflurane. Initial two attempts with direct laryngoscopy (DL) using Miller and Macintosh blades, respectively, revealed a Cormack and Lehane (C&L) grade-IV view. Since bag and mask ventilation (BMV) was adequate, 3 mg succinylcholine was administered. The third attempt by a senior anaesthesiologist using a paraglossal approach with a Macintosh blade and optimum external laryngeal ma-

Corresponding Author: Nishkarsh Gupta E-mail: [email protected] Received: 01.08.2019 Accepted: 18.08.2019 ©Copyright 2020 by Turkish Society of Anaesthesiology and Reanimation - Available online at www.turkjanaesthesiolreanim.org Available Online Date:18.05.2020 Turk J Anaesthesiol Reanim 2020 Gupta et al. Syndromic Children and Videolaryngoscopy

nipulation (OELM) could only improve the view to CLgrade and intubation with styletted 2.5-mm internal diameter (ID) IIIb. The arterial oxygen saturation momentarily reduced to endotracheal tube (ETT) was successful. low 80 s but improved immediately on BMV. The CMAC Miller size ‘0’ blade was then introduced, which resulted in a Case 2 CL grade IIb view that improved to grade IIa with OELM, A 2-day-old male child with Beckwith–Wiedemann syndrome (BWS) was referred to our hospital after normal vaginal deliv- ery for omphalocele repair under GA (Figures 2 and 3). There was a history of hypoglycaemia following birth, and the child was receiving 10% dextrose. Examination revealed peculiar facies, prominent eyes, deformed ears and macroglossia. Mid- line DL with a size ‘0’ Miller blade revealed a CLgrade IIIb view. Another attempt by a senior anaesthetist using a Mac- intosh blade with shoulder roll yielded a CLgrade IIIa view with OELM. The use of Truview-PCD VL (Truphatek In- ternational Ltd., Netanya, Israel), with 5 L min oxygen flow, yielded a grade IIb view that improved to CLgradeI with

Figure 1. Pierre Robin sequence neonate

Figure 3. Beckwith–Wiedemann syndrome neonate being intubated with Truview-PCD VL, and glottic view on the monitor

Figure 2. Beckwith–Wiedemann syndrome neonate

Main Points:

• Airway management using conventional DL may be challenging in cases of syndromic children.

• A series of cases of children with Pierre Robin sequence, Beck- with–Wiedemann syndrome, and Hurler’s syndrome are reported.

• Initial attempts at intubation using DL failed; intubation using VLs succeeded.

• VLs should be used for initial intubation attempts in syndromic children with potential difficult airways. Figure 4. Hurlers syndrome child with foot deformity (inset) Gupta et al. Syndromic Children and Videolaryngoscopy Turk J Anaesthesiol Reanim 2020

and high-arched palate increasing the difficulty in intubation), BWS (large protuberant tongue) and Hurler’s (coarse facies, enlarged tongue, small mandible, large tonsils, large tongue, micrognathia, Mallampati class IV, reduced submandibular compliance, short neck, and limited neck and jaw movement). The problem is further aggravated in those with craniofacial anomalies, especially in emergency scenarios, such as our cases, wherein time for evaluation is limited and waking-up and deferring the case for a later date is not a feasible option.

Airway management in such patients requires careful plan- ning and may need advanced techniques for securing the airway (6). Awake fiberoptic intubation is the safest tech- Figure 5. Hurler’s child (side profile) nique for securing the airway in DA cases but has its limita- tions in this age group, e.g., the lack of cooperation, the un- OELM, and the child was intubated with a styletted 3-mm availability of expertise and/or age-related equipment, and ID ETT. the need for sedation/GA. Therefore, it was not considered in our cases (7). Macroglossia hindered intubation by mak- Case 3 ing it difficult to displace the tongue during laryngoscopy (8). A 10-year-old, male Hurler’s syndrome child with bilater- A DA cart consisting of appropriately sized ETTs, curved al pneumonitis was posted for an emergency incarcerated and straight DLs, oral airways, bougie, supraglottic devices umbilical hernia repair. He had a large head, coarse fea- (SGDs), face masks, VL and fiberoptic bronchoscope (FOB) tures, cloudy cornea, delayed milestones, short stature (106 was arranged. cm), speech deficit and mental retardation (Figures 4 and 5). His parents provided a history of snoring and frequent Sevoflurane inhalational induction with preserved sponta- awakening during sleep. Characteristic foot and rib de- neous respiration is the most favoured technique among pae- formities were present. Airway examination showed short diatric anaesthesiologists for managing DA cases, because the neck, macroglossia, high-arched palate, Mallampati class child’s airway can be established by a graded onset of anaes- IV, enlarged tonsils and poor submandibular compliance. thesia during induction. We managed all the three cases with Sevoflurane-based inhalational induction was planned sevoflurane-based inhalational technique. while preserving spontaneous breaths. The first attempt using a Macintosh blade resulted in a CLgrade IIIb view Muscle relaxants might improve intubation conditions and with OELM. Second attempt with a McCoy blade revealed decrease the incidence of laryngospasm. Hence, after initial grade IIb view; however, since the child started desaturat- failed attempts in the PRC child, we administered succinyl- ing, the attempt was terminated. The BMV was successful choline after ensuring adequate BMV (9). In patients with a only after applying a two-handed technique with the use history of snoring during sleep, BMV may be difficult; there- of oral and nasal airways. Intraoral tissues and pharynge- fore, we avoided muscle relaxants in the Hurler’s child. al space were revealed to be non-compliant and narrow, respectively. Repeat attempt with Truview-PCD VL (with Neonates with PRS have micrognathia (small jaw) and glos- oxygen insufflation of 5 L min–1) resulted in a CLgrade soptosis (the tongue falls to the back of the throat) that leads IIb view with OELM, but intubation (with Optistylet use) to airway obstruction and respiratory distress. Various intu- failed. A 10Fr bougie could be inserted into the trachea, bation techniques including McGrath VL, Airtraq™ VL, and a 5.5-mm ETT could be railroaded over it. FOB and intubation via SGDs have been described; however, CMAC VL has not been reported (10-12). Discussion Airway management of a BWS child may be difficult due This series describes the successful management of difficult to macroglossia hampering with BMV and laryngoscopy airway (DA) in syndromic children using VLs. The anatomi- (13). Glidescope VL has been used to facilitate intubation in cal and physiological differences in paediatric patients make a 4-year-old BWS child with a history of multiple failed at- their airway management challenging. The presence of cra- tempts at intubation; however, the use of Truview-PCD VL niofacial anomalies further aggravated the difficulty in man- has not been previously reported. The Truview-PCD blade agement of airways in the child with PRS (small anterior has a 48º anterior deflection, which helped us visualise the mandibular space, tongue pushed back to the hypopharynx, anteriorly placed larynx (14). Turk J Anaesthesiol Reanim 2020 Gupta et al. Syndromic Children and Videolaryngoscopy

In Hurler’s syndrome, mucopolysaccharides accumulate al-time assessment of airway anomalies and help us modify throughout the body. The infiltration of these deposits in the our plan accordingly. airway leads to macroglossia, thickened and non-compliant soft tissues of the oropharynx and blockage of nasal passag- Conclusion es. This may lead to progressive airway obstruction, difficult BMV and difficult intubation (DI). A review of airway man- VLs have revolutionised the management of paediatric DA. agement in Hurler’s children reported DI in five out of ten At present, the choice of a VL depends on the availability, the children while intubation failed in three. These three cases clinical scenario, individual preference and expertise. Anaes- were rescued by the use of SGD, FOB and Airtraq VL (15). thesiologists should have a multi-layered contingency plan to handle the airway of a syndromic child. Indirect visualisation A multi-centric registry of children with DA demonstrated devices should be used for initial intubation attempts as DL is that more than two DL attempts were associated with high likely to fail in these patients. failure rates and increased complications (transient hypox- emia was the most common) (2). In addition, this study identi- Informed Consent: Written informed consent was obtained from fied that weight <10 kg, micrognathia and three DL attempts the parents of the patients who participated in this study. before indirect technique increased the risk of complications. Our case with Hurler’s syndrome also desaturated despite Peer-review: Externally peer-reviewed. apnoeic oxygenation, probably because of the pre-existing Author Contributions: Concept – A.G., N.G.; Design – A.G., pneumonitis and prolonged intubation attempts. Therefore, N.G.; Supervision – G.K., K.K.G.; Resources – A.G., N.G.; Mate- paediatric anaesthesiologists should minimise the number of rials – A.G., N.G., G.K., K.K.G.; Data Collection and/or Process- DL attempts, use an indirect technique (VL/FOB) early, and ing – A.G.; Analysis and/or Interpretation – A.G., N.G.; Literature consider oxygenation during intubation attempts. Search – A.G., N.G.; Writing Manuscript – A.G., N.G.; Critical Re- view – A.G., N.G., G.K., K.K.G. Although a range of VLs has been introduced, they are yet to be approved as a first-line device for routine intubation in Conflict of Interest: The authors have no conflicts of interest to children. Some studies have shown that VL is better than con- declare. ventional DL for routine or DI in children (4, 5). However, none of them have been carried out in syndromic children. Financial Disclosure: The authors declared that this study has In our cases, early use of a VL could have reduced intubation received no financial support. attempts and multiple failures could have been averted. The CMAC Miller blade comes intuitively to those familiar with References conventional DL. Furthermore, it does not require special preparation and can be used in case of emergency. 1. Holm-Knudsen RJ, Rasmussen LS. Pediatric airway management: basic aspects. Acta Anaesthesiol Scand 2009; 53: 1‐9. [CrossRef] It may be difficult to intubate despite a good glottic view with 2. Fiadjoe JE, Nishisaki A, Jagannathan N, Hunyady AI, Green- VL. Hence, a styletted ETT is recommended for intubation. berg RS, Reynolds PI, et al. Airway management complications Truview VL is provided with its dedicated Optistylet that has in children with difficult tracheal intubation from the Pediatric a fixed curvature, and it may be difficult to direct the ETT. 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